The renal manifestations of systemic lupus erythematosus in childhood range from minor abnormalities detected on urinalysis to severe renal insufficiency requiring renal replacement therapy. Clinically significant renal involvement in systemic lupus erythematosus is more common in children than in adults. Effective treatment for childhood lupus nephritis is available, and the prognosis for affected children has improved over the course of the last 30 years. Corticosteroid therapy remains the cornerstone of treatment for children with lupus nephritis. The addition of cytotoxic agents to corticosteroid treatment improves both the long and short-term prognoses. Cyclosporin may improve the clinical manifestations of lupus nephritis although the disease remains active serologically. Although survival in childhood lupus has improved, complications of therapy result in significant morbidity with distressing frequency. Immunosuppression may result in mortality and morbidity due to opportunistic infections. Individuals with otherwise successful control of renal manifestations of systemic lupus erythematosus may still be left with significant morbidity due to disturbances in growth due to long-term corticosteroid treatment. Psychosocial development may be adversely affected both as a result of chronic illness as well as due to the effects of therapy. Meticulous attention to detail over decades of treatment is necessary to optimize patient outcome in childhood lupus nephritis.